Care of Clients with Problems In Oxygenation, Fluids and Electrolytes, Metabolism and Endocrine (NCM103) Patients With Hematologic

Alterations: Platelets and Clotting Factors

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Normal Values
WBCs: 4,500 11,000 /mm3 Platelets: 150,000 400,000/mm3 Prothrombin Time (PT): 11 12.5s Partial Thromboplastin Time (PTT): 25 35s Bleeding Time: 1.5 9.5 mins D-Dimer 0 0.5 ug/mL

Topics Discussed Here Are: 1. Platelets and Clotting Factors 2. Thrombocytopenia Idiopathic Thrombocytopenic Purpura 3. Hemophilia 4. Disseminated Intravascular Coagulation (DIC) 5. Leukopenia Neutropenia

Alterations of Platelets and Clotting Factors

Platelets
Characteristics: o Formed in the RED BONE MARROW o Tiny cell fragments from MEGAKARYOCYTES o No nucleus o Life Span: Approximately 10 days Functions: o Occlusion of small opening in blood vessel (Hemostatic Function) o Plays a role in blood coagulation (Thromboplastic Function) Mechanism of Hemostasis o Hemostasis is the orderly, stepwise process for stopping bleeding that involves: Vascular Phase:
Vasoconstriction of Blood Vessel Diameter of Blood Vessel Blood Volume

Platelet Plug Formation: Aggregation of Platelets Collagen stimulates Platelets Coagulation: Extrinsic and intrinsic factors Fibrinolysis or Clot Dissolution The FINAL PHASE is Fibrinolysis or Clot Dissolution

Seal off injury

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Platelets and Clotting Factors

For Normal Clotting and Lysis To Occur: - Intact Blood Vessels - Adequate platelets - Sufficient amount of the 12 clotting factors - Well-controlled fibrinolytic system Bleeding disorders are diagnosed based on: o Complete health history o Physical exam o Laboratory tests for platelets and clotting factors
Lab Studies provide the most crucial data to pinpoint the cause of hemorrhagic disease

Signs and Symptoms o Petechiae Tiny hemorrhagic spots caused by intradermal or subcutaneous bleeding o Ecchymoses Large, blotchy subcutaneous hemorrhagic area o Hematoma Subdermal hemorrhage o Hemarthrosis Bleeding in the joints

Thrombocytopenia
The most common cause of hemorrhagic disorders Decrease in circulating platelets (Less than 100,000/uL) 4 Major reasons why Thrombocytopenia occurs: o in platelet production o platelet lifespan o Blood pooling of spleen o Dilution of the blood stream 50,000 uL = Minor trauma leads to bleeding 10,000 uL = spontaneous bleeding even without trauma

TYPES of Thrombocytopenia
1. 2. 3. 4. Immune / Idiopathic Thrombocytopenic Purpura Thrombotic Thrombocytopenic Purpura Secondary Thrombocytopenic Purpura Disordered Platelet Distribution

Immune Thrombocytopenic Purpura

Idiopathic thrombocytopenic purpura (ITP) Hemorrhagic auto-immune disease that results in destruction of platelets Etiology and Risk Factors o Binding of auto-antibodies to antigens on platelet membrane o Common among: Women: 20-40 years old Children: After a viral infection onset

Pathophysiology

Deadly Dilemmas: o Spontaneous hemorrhage, cerebral, GIT, GUT, diaphragm o Nerve pain, extremity anesthesia and paralysis

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Signs and Symptoms: (G-SHEEEP) o Gingival Bleeding o Splenomegaly (Tend to eat foreign platelets) o Heavy menses (Menorrhagia) o Ecchymosis o Epistaxis o Easy bruising o Petechiae Diagnosis: o Platelet count below 100,000 uL o Prolonged bleeding time with normal coagulation time o Capillary fragility o (+) Platelet antibody screening o Bone marrow aspirate containing normal or number of megakaryocytes Medical Management: o Goal: Safe Platelet COUNT! o High-dose corticosteroids SE: Moon-faced, buffalo-hump, susceptible to stress Suppresses platelet destruction o Plasmapheresis (Short-term therapy) o IV Gamma Globulin ( Platelet count) o Splenectomy 60 80% results in complete / permanent remission o Immunosuppressive Drugs Vincristine Vinblastine Azathioprine Nursing Management: o Health Teaching Signs of disease exacerbation SE of drugs being taken by client o Prevention of bleeding Soft bristle toothbrush Electric shavers Care with trimming nails Avoid rectal temperature taking, enemas and suppositories Avoid IM injections Avoid activities that ICP Avoid sexual intercourse if platelet count is <10,000 mm3 If for venipuncture, draw all blood for lab work with one venipuncture

Hemophilia
X-linked genetic bleeding disorder Deficiency of coagulation factors Found usually among males Detected during early childhood, usually in toddler age (bleeding at 1 y/o)

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Types
Hemophilia A Classic Factor VIII Hemophilia B Christmas Disease Factor IX von Willebrands Disease o vWF carries factor VIII into the circulation and promotes platelets function

Classification
Severe = <1% of normal factor level (spontaneous hemorrhage) Moderate = <1% - 5% Mild

Manifestations Bleeding (HIS-SHEP) o Hemarthrosis o Internal Bleeding o Surgical procedure that result in excessive bleeding o Spontaneous hematuria o Hematoma o Epistaxis, bleeding of other mucous membranes o Pain Medical Management Fresh frozen plasma (contains clotting factors) Factor VIII and IX concentrates o During acute bleeding, preventive measure when undergoing procedure Aminocaproic acid o Fibrinolytic enzyme, slows the dissolution of clots Desmopressin o Vasopressin analog o Induces transient rise in factor VIII Nursing Management Applying pressure on minor trauma Splinting with joint or muscle hemorrhages Avoid injections and all other invasive procedures Perform only after administration of appropriate factor replacement Analgesics for pain Warm bath may improve and lessen pain o During hemorrhagic episodes, heat can accentuate bleeding o Cold compress used Wear necessary medical band for identification During hemorrhagic episodes o Careful assessment for emergent complications o Frequent VS monitoring o WOF: Respiratory distress and altered LOC Psychological support: Assist patient in coping and accepting condition Health teaching: o Activity restriction o Self-care measures o Safety at home and workplace o Avoid any agents that interfere with platelet aggregation (aspirin, NSAIDS, etc) o Dental hygiene

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Disseminated Intravascular Coagulation (DIC)

Complex syndrome of activated coagulation Loss of balance between the clotting and lysing system of the body Consumptive coagulopathy DIC starts with excessive clottings o Clotting factors depleted o Excessive bleeding ensues Categories of RISK Factors: (STIDS) o Secondary response to primary insult o Tissue coagulation factors o Infection (most common cause) Gram (-) septicemia Typhoid Fever Rocky mountain spotted fever o Damage to vascular endothelium o Stagnant blood flow Conditions that may precipitate DIC o Shock o Cirrhosis o Snake bite o Glomerulonephritis o Septicemia o Retention of a dead fetus o Trauma o Heat stroke Pathophysiology
Balance of Coagulation

Precipitating Event (e.g. Infective organ)

Tissue Factor Release

Coagulation cascade

Excess thrombus

Conversion of Plasminogen to Plasmin

Microvascular Clotting Thrombocytopenia

Macrovascular Clotting Consumption of Clotting Factors Excess Bleeding Fibrinolysis with FDPs

Excess Clotting

Ischemia Impaired organ perfusion End Organ Damage

Shock Hypotension Increased Vascular Permeability

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FOR DIC

Test
Platelet Count Prothrombin Time Partial Prothrombin Time Thrombin Time Fibrinogen D-Dimer Fibrin Degradation Products Management o Goals:

Function Evaluation
Platelet Number Extrinsic Pathway Intrinsic Pathway Clot formation Amount available for clot Local Fibrinolysis Fibrinolysis

Changes in DIC
Decreased Increased Increased Increased Decreased Increased Increased

Identification and correction of precipitating cause or problem Reestablishing Hemostasis by replacing missing blood components (Depends on type) Supportive therapy to control manifestation of hemorrhage and thrombosis Control bleeding Gabexate and aprotinin IV Heparin administration Used to control thrombosis May also cause bleeding To dissolve the clot Nursing Management o Assess all body systems Integumentary Respiratory: Tachypnea, Hemoptysis, Orthopnea, pulmonary infarction Cardio: Tachycardia and hypotension GI: Abdominal distension, hematemesis (+), guaiac test (Test for blood in stool) GUT: Hematuria, Oliguria Neuro: Vision changes, dizziness, HA, changes in mental status General Goals Monitor and quantify blood loss Provide supportive therapy with blood components Resolve manifestations of hemorrhage Control further bleeding

Alterations in Leukocytes
Leukocyte Mobile units of the body; protective system Formed in: Bone marrow and lymph nodes Function: o Seek out and DESTROY o Chemotaxis o Phagocytosis Cellular ingestion of the offending agent Types: 1. Granular Basophil, Eosinophil, Neutrophil 2. Non-Granular/Mononuclear Leukocytes - Lymphocytes - Monocytes

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Types of Granulocytes (BEN) Normal Values Basophils 0 0.7%

Eosinophils

0.5 1%

Neutrophils

55 70%

Features / Functions IgE receptors Histamine Heparin Life span = 8 12 Days E(a)limentary and respiratory tract E(a)llergy Parasitic infection * asthma = Eosinophils 1st line of defense against bacterial infection Neutrophils = Infection!

Alterations in Leukocytes
Divided into: Non-neoplastic Disease o Quantitative Disorder Neoplastic Disorders of Lymphoid Organs and Hematopoiesis o o Lymphoma o Leukemia

Leukopenia
A condition in which there are a fewer leukocytes than normal results from Neutropenia or Lymphopenia Neutropenia o Acute potential blood dyscrasia o Failure to produce adequate numbers of Neutrophils (Less than 2000/mm3) o Females are more susceptible than males o Susceptible to bacterial invasion and infection o Causes: Production of neutrophils Aplastic anemia (due to medications/toxins) Metastatic cancer lymphoma leukemia Myelodys plastic syndromes Chemotherapy Radiation therapy Destruction of neutrophils Hypersplenism (Splenomegaly) Medication-induced Immunologic disease (SLE) Viral disease (Ex. Infection, hepatitis, mononucleosis) Bacterial infections o Clinical Manifestations Rapid onset Severe fatigue and weakness Sore throat Buccal and pharyngeal ulcerations Abdominal discomfort Fever Weak and rapid pulse Severe Chills -

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Nursing Management (Interventions) Assess for fever, q4 hours and for hypothermia Hand wash prior any patient contact NC count below 500 /mm3 protective isolation Anyone with URTI are not allowed to see the patient All live plants and flowers are not allowed inside the patients room Low bacteria diet Assist in daily bath Meticulous oral hygiene Perineal cleansing Daily stool softeners Avoid catheter insertion, suppositories, rectal thermometers Other Management Removal of causative factor Disease is usually reversed 2 3 weeks after BMT may be required if condition is not reversed Surveillance cultures of blood, throat, sputum, urine and stool To monitor infections Treatment includes colony-stimulating factors (CSF) to Neutrophil production Given after offending agent is eliminated Given before serious infection, prognosis is better Broad spectrum antibiotics Control of oral and gingival pain Meticulous oral care Saline rinses Local anesthetic gels and gargles Soft or liquid food until mouth and gum sores are diminished

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